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Hypogonadism, Hypogonadotropic

definition

Congenital and familial disorder characterized by hypogonadotropic hypogonadism, eunuchoidal features, and anosmia or hyposmia. It is caused by a defect in the synthesis and/or release of LHRH (luteinising hormone-releasing hormone; GONADORELIN) from the hypothalamus as a result of faulty embryonic neuronal migration. The gene responsible for this is found in the region Xp22.3 on the short arm of the X chromosome. There is a homolog on the Y chromosome. The syndrome is more prevalent in males by a ratio of three to one.